About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Wisconsin Creutzfeldt Jakob Disease, CWD, TSE, PRION REPORTING 2011

MADISON – State wildlife officials will conduct sampling and testing of hunter-harvested deer for chronic wasting disease primarily within the disease management zone of in southern Wisconsin during the 2011 deer season.

“Our goal is to continue to track trends in disease prevalence and distribution and to assess the impacts of CWD management,” said CWD Assistant Coordinator Tim Marien.

Mandatory sampling of adult deer will take place in the western (parts of Dane and Iowa counties) and eastern (parts of Rock and Walworth counties) monitoring areas, and within an 84 square-mile area that encompasses Devil’s Lake State Park. Active surveillance utilizing solicited but voluntary sampling will also be conducted in the area surrounding the western monitoring area in parts of Dane, Iowa, Richland, and Sauk counties.

Surveillance a tool in disease management

Surveillance – the sampling and testing of deer for disease – is one of the key components of DNR’s disease management strategy, according to agency biologists and researchers.

“It is important that we know where the disease is and what it is doing for containment efforts to be effective,” Marien said.

This year, DNR biologists will implement new strategies aimed at optimizing the “efficiency and efficacy of detecting changes in the location and trends in prevalence of this disease” in the management zone, Marien said.

Additionally, DNR will be implementing a pilot program to potentially replace the current system of regional, intensive-surveillance sweeps conducted statewide every five years with a new low-level annual surveillance plan. This plan will focus surveillance on adult deer because, if CWD is in an area, adult deer are the ones most likely to have it. This pilot program will take place in Waupaca, Waushara, Waukesha, Milwaukee, Racine, Kenosha, Buffalo, and Burnett counties.

Wildlife biologists will also be asking hunters to submit deer for sampling from areas around two former deer farms in Portage County where CWD has previously been found in captive cervids.

The Hunt.Harvest.Help CWD outreach campaign is now in full swing. Working with landowners and hunters from the CWD-MZ and a professional communications firm, the DNR developed this campaign to help inform Wisconsin residents about the details of Wisconsin’s CWD Response Plan and what they can do to help. Hunt.Harvest.Help is focused on promoting the fact that in order to successfully manage CWD, DNR, hunters, and landowners will have to work together as a team. Learn more about these efforts, about what CWD is doing out west, and more at [www.knowcwd.com] (exit DNR).

MADISON – State wildlife officials and conservation wardens are reminding hunters that baiting and feeding of deer is banned in 28 Wisconsin counties and that current law places restrictions on the timing and amount of bait that can be used for hunting purposes in all remaining counties. Additional information on baiting and feeding regulations can be found in the 2011 Deer Hunting regulations (pdf).

Wildlife health officials say chronic wasting disease is transmitted through deer to deer contact, so baiting is banned in the CWD zone. Concentrations of deer at bait and feeding sites are likely to promote the transmission of infectious agents. CWD is also transmitted through exposure to a contaminated environment. Scientific studies have concluded that CWD, the always fatal disease in deer, can be spread through saliva passed between deer at baiting and feeding locations.

Practices impact deer behavior

“Deer hunters know that baiting and feeding decrease deer movement which reduces deer sightings and hunting opportunities away from the baiting site,” said acting DNR big game biologist, Dan Hirchert. “These practices can also draw deer into residential areas where hunting may be prohibited or restricted”

Officials: “We’re making progress”

Illegal baiting and feeding are among the most frequent violations cited by conservation law enforcement officers and one of the most frequent sources of citizen complaints according to law enforcement officials. However, awareness of baiting and feeding restrictions and impacts is growing through outreach and education.

In 2010, the top violation encountered by conservation wardens during the gun deer season was illegal baiting of deer. But there is promising news. The number of arrests for illegal baiting at 216 represented a 35 percent decline from 2009

The 2010 deer season report said complaints to the DNR Hotline regarding illegal baiting and feeding also were down 50 percent. The volume of material found in bait piles also dropped.

MADISON – Hunters and landowners can learn more about what they can do to maintain a healthy deer herd and Wisconsin’s strong hunting traditions through a new website dedicated to sharing information on Chronic Wasting Disease.

The website, www.knowcwd.com, carries the theme of “Hunt. Harvest. Help” and features racing champion Matt Kenseth, a deer hunter and Cambridge, Wis., native, in a public service announcement talking about the importance of teamwork in tackling CWD.

"As a deer hunter, I'm concerned about CWD," Kenseth says in a video public service announcement on the website. "But it's going to take more than one person to slow the spread of CWD…It's a team effort Wisconsin. So get out there and hunt, harvest and help."

Hunt, Harvest, Help. WDNR Photo

Department of Natural Resources wildlife officials say the website was developed to share information on how CWD is spread, where the disease exists in the Wisconsin deer population and what other states with CWD are doing about it. There also is information about human health risks. Several additional tabs on the website direct visitors to information on how individuals can help, frequently asked questions and videos.

The website also links to important CWD management information including Wisconsin’s CWD Response Plan and current and past CWD research and statistics.

“CWD has the potential for significant, negative impacts on the future of deer and deer hunting anywhere it exists,” said Davin Lopez, DNR’s CWD coordinator. “Minimizing the area of Wisconsin where the disease occurs is the responsible thing to do. Wisconsin’s current CWD policy is containment, rather than elimination of the disease. Hunter and landowner participation is key to this effort.

Beginning the week of Aug. 15 TV viewers in the CWD management zone will see CWD public service announcements featuring Kenseth. Also the "Hunt. Harvest. Help." theme will appear on billboards, in print ads and in other online sources.

The website and materials were developed with the aid of a U.S. Department of Agriculture/Veterinary Services grant and a private sector communications firm.

Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts --- Wisconsin, 2002

Creutzfeldt-Jakob disease (CJD) is a fatal neurologic disorder in humans. CJD is one of a group of conditions known as transmissible spongiform encephalopathies (TSEs), or prion diseases, that are believed to be caused by abnormally configured, host-encoded prion proteins that accumulate in the central nervous tissue (1). CJD has an annual incidence of approximately 1 case per million population in the United States (1) and occurs in three forms: sporadic, genetically determined, and acquired by infection. In the latter form, the incubation period is measured typically in years. Recent evidence that prion infection can cross the species barrier between humans and cattle has raised increasing public health concerns about the possible transmission to humans of a TSE among deer and elk known as chronic wasting disease (CWD) (2). During 1993--1999, three men who participated in wild game feasts in northern Wisconsin died of degenerative neurologic illnesses. This report documents the investigation of these deaths, which was initiated in August 2002 and which confirmed the death of only one person from CJD. Although no association between CWD and CJD was found, continued surveillance of both diseases remains important to assess the possible risk for CWD transmission to humans.

Case Reports

Case 1. In December 1992, a Wisconsin man aged 66 years with a history of seizures since 1969 sought treatment for recurring seizures, increasing forgetfulness, and worsening hand tremors. Electroencephalographic (EEG) examination demonstrated focal epileptiform activity and nonspecific diffuse abnormalities, but no specific diagnosis was made. In February 1993, he was hospitalized for increasing confusion, ataxia, and movement tremors of his extremities. A magnetic resonance image (MRI) demonstrated mild, nonspecific enhancement along the inferior parasagittal occipital lobe. A repeat EEG showed bifrontal intermittent, short-interval, periodic sharp waves, suggesting a progressive encephalopathy; a diagnosis of CJD was suspected. The man died later that month; neuropathologic examination of brain tissue during autopsy indicated subacute spongiform encephalopathy, compatible with CJD.

The man was a lifelong hunter who ate venison frequently. He hunted primarily in northern Wisconsin but also at least once in Montana. He hosted wild game feasts at his cabin in northern Wisconsin from 1976 until shortly before his death. Fixed brain tissue obtained during the autopsy was sent for analysis to the National Prion Disease Pathology Surveillance Center (NPDPSC) and reexamined at the institution where the autopsy was conducted. Histopathologic examination did not substantiate the diagnosis of prion disease. In addition, 27 brain tissue sections were negative for prions by immunostaining despite positive antibody reactions against other proteins (controls), which indicated that other epitopes in the tissue samples were preserved.

Case 2. In May 1999, a Minnesota man aged 55 years with no previous history of a neurologic disease sought evaluation and treatment following a 3-month history of progressive difficulty in writing and unsteadiness of gait. A computerized tomography (CT) scan and MRI examination of his head did not indicate any abnormality. In June 1999, he was hospitalized following onset of dementia, speech abnormalities, and myoclonic jerking. An EEG indicated left-hemispheric periodic sharp waves and moderate generalized background slowing; CJD was diagnosed clinically. In July 1999, following worsening symptoms and development of right upper extremity dystonia, the patient died. Neuropathologic evaluation of brain tissue during autopsy demonstrated widespread subcortical spongiform lesions, consistent with CJD.

The man was not a hunter but had a history of eating venison. He made an estimated 12 visits to the cabin where the wild game feasts were held, but he participated in only one feast during the mid-1980s. Sections of fixed and frozen brain tissue obtained during autopsy were analyzed at NPDPSC, and prion disease was confirmed by immunohistochemical and Western blot testing. The Western blot characteristics and prion disease phenotype in this patient were consistent with the most common form of sporadic CJD, classified as M/M (M/V) 1 (3). Subsequent genetic typing confirmed the presence of methionine homozygosity (M/M) at codon 129 of the patient's prion protein gene.

Case 3. In June 1992, a Wisconsin man aged 65 years sought treatment for progressive slowing of speech, worsening memory, and personality changes. By January 1993, his speech was reduced to one-word utterances. Neurologic examination showed a flat affect, decreased reflexes, and apraxia. A CT head scan showed mild atrophy, and an EEG was normal. Pick's disease was diagnosed. By May, he was unable to perform any daily living activities; he died in August 1993. Neuropathologic evaluation of brain tissue during autopsy showed symmetrical frontal lobe cerebral cortical atrophy and mild temporal lobe atrophy. No Pick's bodies or spongiform lesions were observed.

The man had a history of eating venison and participated regularly in wild game feasts held at the cabin owned by patient 1. He was a lifelong hunter and hunted mostly in Wisconsin but also in Wyoming and British Columbia. No game was brought to the wild game feasts from his hunting trips outside of Wisconsin. Examination of fixed brain tissue sent to NPDPSC demonstrated no lesions indicative of CJD, and immunohistochemical testing with antibody to the prion protein did not demonstrate the granular deposits seen in prion diseases.

Epidemiologic Investigation

Wild game feasts consisting of elk, deer, antelope, and other game that occurred at a cabin in northern Wisconsin owned by patient 1 began in 1976 and continued through 2002. These feasts typically involved 10--15 participants and usually occurred on weekends before or during hunting seasons in the fall and occasionally in the spring. Wild game brought to these feasts usually were harvested in Wisconsin, but three men who attended these feasts reported hunting in the western United States and bringing game back to Wisconsin. These activities took place in Colorado (near the towns of Cortez, Trinidad, Collbran, Durango, and Meeker), Wyoming (near the towns of Gilette and Cody), and Montana (near the town of Malta). CWD was not known to be endemic in these areas at the time that these hunting activities took place.

Information was obtained for 45 (85%) of 53 persons who were identified as possibly participating in the wild game feasts; all were male. Information was obtained by direct interview or from family members of decedents. Of the 45 persons, for whom information was obtained, 34 were reported to have attended wild game feasts. Seven of the 34 feast attendees were deceased, including the three patients. None of the four other decedents had a cause of death attributed to or associated with a degenerative neurologic disorder. None of the living participants had any signs or symptoms consistent with a degenerative neurologic disorder.

CWD was first described in the United States in the 1960s and classified as a TSE in 1978. Previously localized to a contiguous endemic area in northeastern Colorado and southeast Wyoming, since 2000, CWD has been found in free-ranging deer or elk in Illinois, Nebraska, New Mexico, South Dakota, Wisconsin, and outside the previously known endemic areas of Colorado and Wyoming. CWD has been identified also in captive deer or elk in Colorado, Kansas, Minnesota, Montana, Nebraska, Oklahoma, South Dakota, and Wisconsin (4). Because a variant form of CJD, with specific neuropathologic and molecular characteristics that distinguish it from sporadic CJD, has been associated with eating cattle products infected with a prion that causes bovine spongiform encephalopathy (5), concern has been raised about the possibility that the prion associated with CWD might be transmitted to humans in a similar way.

In this investigation, because only one of the three cases in Wisconsin had neuropathologic confirmation of a prion disease, no association could be made between case participation in the wild game feasts and the development of CJD. Although patient 2 had confirmed CJD, he was unlikely to have eaten CWD-infected venison at these feasts because venison and other game from outside Wisconsin that was served at these feasts did not originate from known CWD-endemic areas, and the man participated in the feasts only once. In addition, the prion disease in this case was consistent with the most common form of sporadic CJD, without apparent unusual neuropathologic or molecular characteristics that might occur if the prion related to CWD had been responsible for the disease.

The findings in this report are subject to at least two limitations. First, not all members participating in wild game feasts could be identified, and not all persons listed as participating could be contacted for interviews. Second, interviews that were conducted required recall of events that occurred up to 25 years ago, limiting the detail or accuracy of events. However, the similar responses obtained from different sources support the accuracy of the investigation findings.

A previous investigation of unusually young CJD patients in whom the transmission of CWD was suspected also did not provide convincing evidence for a causal relationship between CWD and CJD (2). However, limited epidemiologic investigations cannot rule out the possibility that CWD might play a role in causing human illness. Ongoing surveillance of CJD, particularly in states with CWD, is important to assess the risk, if any, for CWD transmission to humans. Because the confirmation of CJD and the detection of a new prion disease require neuropathologic study of brain tissue, physicians are encouraged to contact NPDPSC (http://www.cjdsurveillance.com; telephone, 216-368-0587) to confirm diagnoses of CJD and to distinguish its various subtypes. Because of the known severity of TSEs in humans and the possibility that the CWD prion can affect humans, animals with evidence of CWD should be excluded from the human food or animal feed chains. Hunters and wild venison consumers should follow precautionary guidelines available from the Wisconsin Department of Agriculture, Trade, and Consumer Protection (http://datcp.state.wi.us/core/consumerinfo) to prevent potential exposures to the CWD agent.

Investigators find no common source in hunters' deaths 2 of 3 show absence of prions By JOHN FAUBER and LEE BERGQUIST jfauber@journalsentinel.com Last Updated: Nov. 21, 2002

Three hunters who ate wild game together and later died of rare brain disorders did not contract their diseases from a common source such as venison, a four-month-long investigation concluded Thursday. [19335] Chronic Wasting Disease For complete archived coverage of chronic wasting disease in Wisconsin, go to our SPECIAL SECTION Quotable The idea here is that there is no fear now. These cases are one of the things that stopped many spouses, or hunters from hunting, because it sounded so plausible - and now it is completely debunked. - Fred Bannister, Chetek physician who attended many game dinners with the late Wayne Waterhouse Related Coverage Deer hunting: Season starts saturday Section: Outdoors Section: Chronic wasting disease

A report by federal and state health investigators found that one of the men apparently had been misdiagnosed with Creutzfeldt-Jakob disease, a neurological disorder caused by prions. Prions are the same unusual infectious agents that cause chronic wasting disease in deer and elk.

Pathologists were able to run new tests of brain tissue from Wayne Waterhouse of Chetek, who died in 1993, and determined that there was no evidence of a prion-related illness, said Jeffrey Davis, the state epidemiologist for communicable diseases.

Health officials said they did not know what brain disorder killed Waterhouse, an avid hunter and outdoorsman.

In September, health officials said new test results from brain tissue samples of another man, Roger Marten of Mondovi, also showed no evidence of prions or Creutzfeldt-Jakob disease. A third man, James Botts of Minneapolis, did die of the disease, the new analysis of his tissue confirmed.

"These results are important because if all three men had developed a rare disease like CJD (Creutzfeldt-Jakob disease), such a cluster would suggest a common source of exposure," Davis said. "Thanks to a new testing process not available at the time of the initial diagnosis of CJD in these patients, we were able to demonstrate the absence of prions in brain tissues of two of the patients."

The new analysis, part of a joint investigation by the Wisconsin Division of Health and the U.S. Centers for Disease Control and Prevention, contradicts a 1993 diagnosis made at the Mayo Clinic, where Waterhouse died.

The new report could buoy the confidence of deer hunters. The report was issued two days before the start of the 2002 gun deer season.

The hunt is considered the most important in decades because of a plan by the state Department of Natural Resources to eradicate 25,000 deer in a 411-square-mile region of Dane, Iowa and Sauk counties to control the spread of chronic wasting disease.

The disease has prompted questions about the safety of venison and has helped drive down the number of deer hunters buying licenses this year.

"From a hunters' standpoint, (the report) may give hunters a little more confidence about consuming venison," said Darrell Bazzell, secretary of the DNR.

Sales of deer-hunting licenses have picked up in the past few weeks, but as of Wednesday sales lagged 16% behind the same time last year, according to the DNR. So far this year, there have been 118,441 fewer licenses sold.

Bazzell said the new findings could give an additional last-minute boost to license sales.

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

The center receives federal funding to monitor prion diseases, including the human version of mad cow disease, and is considered one of the top prion labs in the country.

Davis said it was his understanding that pathologists at the lab concurred with pathologists at Mayo and also were in agreement on the new analysis of Waterhouse's tissue.

The case of the three outdoorsmen was first reported in the Journal Sentinel in July and has since attracted widespread attention and heightened concerns about the safety of venison.

Waterhouse, Marten and Botts, a former Chetek resident who later moved to Minneapolis, all had eaten wild game at the Waterhouse family cabin on the Brule River in northern Wisconsin.

Chetek physician Fred Bannister, who attended many of the game dinners with Waterhouse, emphasized that no deer from the dinners came from the 411-square-mile eradication zone.

Bannister, also a deer hunter, said he had been waiting for scientific corroboration that his friend had not died of Creutzfeld-Jacob disease.

"How can you have good news about someone who died?" Bannister said. "But the idea here is that there is no fear now. These cases are one of the things that stopped many spouses, or hunters from hunting, because it sounded so plausible - and now it is completely debunked."

However, Judy Botts, wife of James Botts, said the new findings had not changed her mind.

Botts still suspects that her husband's consumption of venison played a role in his death, "but it can't be proven," she said. "I knew it all along."

Botts died in 1999. New analysis of his tissue confirmed his diagnosis of Creutzfeldt-Jakob disease, which occurs at the annual rate of about one per million people.

Marten died in 1993. He initially was diagnosed with Pick's disease, another rare brain disease. A new analysis of his brain tissue found that although he did have Pick's, he did not have a prion disease.

About 75 men were known to attend the wild game feasts, Davis said. Investigators have been able to contact about 45 of them. No other case of rare brain disease has been found.

Davis said the new findings were consistent with earlier statements by health organizations that chronic wasting disease prion "has not been shown to cause human illness."

Some neurologists and prion researchers have cautioned that the question of whether chronic wasting disease can jump to people remains unanswered.

Some have said that until proved otherwise, it is reasonable to assume that the disease may jump to people in a manner similar to mad cow disease. Mad cow disease is believed to have caused at least 130 cases of variant-Creutzfeldt-Jakob disease, an always fatal disorder, in about 130 people, mainly in Great Britain.

The new information could entice some wavering hunters to head into the woods, said David Ladd, chairman of the Big Game Committee of the Conservation Congress, a citizens group that advises the DNR.

But Ladd said the discovery of chronic wasting disease had altered the psyche of the 2002 hunt.

"A lot of hunters are probably not going to make a decision until they pull the trigger," he said.

A version of this story appeared in the Milwaukee Journal Sentinel on Nov. 22, 2002.

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

The center receives federal funding to monitor prion diseases, including the human version of mad cow disease, and is considered one of the top prion labs in the country.

SNIP...

$$$

TSS

11/21/02, LAB FINDINGS RELEASED ON FATAL CASES OF NEUROLOGIC DISEASES IN OUTDOORSMEN

(MADISON ? November 21, 2002) -- The Wisconsin Division of Public Health today released completed test results related to the investigation into the fatal cases of degenerative neurological illnesses in three men who consumed wild game served during a series of feasts.

The test results announced today indicate that Wayne Waterhouse, a northern Wisconsin resident who died in 1993, did not have Creutzfeldt-Jakob disease (CJD) or any other evidence of prions or prion-related illness. Results already made public indicated that another of the patients, Roger Marten, a northern Wisconsin resident who died in 1993, also did not have CJD or any other evidence of prions or prion-related illness. Only one of the three men, James Botts, a Minnesota resident who died in 1999, had a confirmed diagnosis of CJD.

"These results are important because if all three men had developed a rare disease like CJD, such a cluster would suggest a common source of exposure," said Dr. Jeffrey Davis, Wisconsin State Epidemiologist for Communicable Diseases, in reporting the findings. "Thanks to a new testing process not available at the time of the initial diagnosis of CJD in these patients, we are able to demonstrate the absence of prions in the brain tissues of two of the patients. Therefore, these three cases cannot be attributed to a common source of illness."

The current investigation, conducted by the Division of Public Health and the U.S. Centers for Disease Control and Prevention, was initiated after reports surfaced that rare degenerative neurological diseases had occurred in three acquaintances who shared meals of wild game in northern Wisconsin. The reports generated considerable public interest due to the concern that these illnesses might somehow be linked to chronic wasting disease (CWD) of deer and elk.

"These findings are consistent with earlier statements by the CDC and the World Health Organization that the CWD prion has not been shown to cause human illness," Dr. Davis added. "They also illustrate the ongoing need to apply the most current scientific techniques to the important issue of understanding CWD and other prion-related conditions."

Specimens of brain tissue from each of the three men had been collected during the individuals? autopsies. The tissues were recently forwarded to the National Prion Disease Pathology Surveillance Center in Cleveland, Ohio, where pathologists examined the specimens for evidence of CJD and the presence of abnormal prion proteins which cause the illness. This pathology center was established during 1996-1997 by CDC in collaboration with the American Association of Neuropathologists to enable state-of-the-art laboratory investigation of physician-diagnosed and suspected cases of prion disease in the United States.

Creutzfeldt-Jakob disease is a fatal degenerative brain condition of humans believed to be caused by an abnormally-shaped protein called a prion. It occurs at a rate of about one case per million people per year throughout much of the world, and was first described in the 1920s. Chronic wasting disease in deer and elk is also believed to be caused by a prion and produces lesions in the brain, but the deer CWD prion has not been shown to affect humans.

Dr. Davis reiterated that while these new results are reassuring, it is still impossible to say with absolute certainty that the CWD prion will never cause human illness. As a precaution, he continues to advise that hunters process their venison in a safe manner and not ingest tissues where the CWD prion is known to concentrate. These tissues include brain, spinal cord, eyes, lymph nodes and spleen. The Wisconsin Department of Agriculture, Trade, and Consumer Protection has issued recommendations on processing deer. These can be found on their website at

how about a statement from Gambetti et al, instead of a bunch of alleged second hand statements.

does state epidemiologist or even mayo really have access to a specialized panel of _proven_ antibodies and know how with prion ihc??? probably not.

only gambetti and prusiner have the necessary reference collections if i am not mistaken $$$

who validated it, false positive, false negatives?

what's going on here???

just in time too to avert a public panic and at the opening weekend of deer hunting season at that, how convenient and excellent timing$$$

unpublished, unpeer-reviewed = unreliable

and just more BSeee.

and what did deep throat tell me many moons ago;

..."I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at....."

snip...

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

i sure would be interested to know more about this new test they are using now, and why it has not been used in the past, or were they just waiting for the opening weekend of deer season? so many things to ponder$

all these new test (cjd now, CWD, Scrapie), but absolutely nothing on any sort of rapid testing TSE 1 M cattle annually in the USA$$$ (more convienience)

i have serious doubts about the _new_ CJD Foundation and their intentions. you know the one, the one that _refuses_ to make up a CJD Quesionnaire asking questions to seek answers as to the route and source of sporadic CJDs in the USA, the same one funded by the CDC??? i have one email from a family member of a very young CJD victim in the USA (sporadic of course) that wrote me questioning the fact that the _new_ CJD Foundation/CDC questionnaire was useless. the family wrote telling them more info on there own, cause they said there were not many questions being asked. see comments snip;

I also sent xxxx a follow-up email with more info because I didn't think the questionnaire asked enough, or perhaps I just needed to write about it. I sent it on xxxx. See next...

snip...

also;

i am seeing more and more confirmed sporadic CJDs, that are being negated by the _new_ CDC funded CJD Foundation? see ref;

(this was the one where they changed the diagnosis of CJD to just dementia, from after death analysis of only a small diamond shape piece from back of brain. the victim could not sleep for 3 to 4 days at a time etc. the family told me that the original diagnosis was made from MRI and spinal, before being changed by CJD Foundation/CDC to only dementia.)

now i am back to here;

Thursday's report contradicts the findings of a Mayo Clinic doctor, who in a May 17, 1993, letter to Waterhouse's family said a postmortem exam of Waterhouse confirmed that he died of Creutzfeldt-Jakob disease.

In fact, when reached at home on Thursday night, Joseph Parisi, a Mayo pathologist, said the new tests on Waterhouse were "inconclusive" and could not confirm that he died of the disease.

That differs from a statement issued Thursday by Davis' office, which says that Waterhouse did not have Creutzfeldt-Jakob disease. Davis also said the latest analysis was confirmed by pathologists at the National Prion Disease Pathology Surveillance Center in Cleveland.

The center receives federal funding to monitor prion diseases,

snip...

what's going on here??? heck, just more of my conspiracy theories i suppose...

not picks, not cjd ???

CWD and neurological disease cluster link investigated

Mary Quirk

No link to chronic wasting disease (CWD) is suggested from the re-examination of one case in a cluster of degenerative neurological diseases involving three men who participated in wild game feasts, according to public health officials.

Of one group of 75 people who regularly attended these annual meals in the US midwest since the late 1970s, 43 have been contacted and interviewed. "We are not aware of any other related health events in this group", Jeffrey Davis (Wisconsin Division of Public Health, Madison, WI, USA) told TLID.

Brain autopsy samples from the three men were sent to the National Prion Disease Pathology Surveillance Center (NPDPSC) in Cleveland, OH, USA. The centre examines an estimated 50-60% of Creutzfeldt-Jakob disease (CJD) cases in the USA, according to Shu G Chen, director of their Prion Protein Analysis Laboratory. Archived tissues may be re-examined to confirm prion disease, and to conduct up-to-date immunohistochemistry and prion protein genetic testing.

Until now findings have been reported on one case. NPDPSC pathologists have concluded that, although they did not concur with the previous diagnosis of Pick's disease, a form of dementia, the autopsy tissue from 1993 showed no evidence of a prion-related disorder. Information is still pending on the other two, both suspected cases of CJD; one man died in 1993 and the other in 1999.

Davis has participated in CWD information meetings for the public in Wisconsin, where he focuses on current knowledge about CWD, recommending that residents heed WHO precautions, and directs hunters to advice on the internet

At the feasts, people shared game that they had harvested throughout the USA. "We are not aware of any meat having come from known CWD-endemic areas", says Davis.

Previously, researchers have reviewed CJD cases from CWD-endemic areas to look for differences in clinical symptoms, pathology, or the characteristics of the prion protein. "So far, we haven't found a link", Chen told TLID. "For us to be able to tell whether these cases would have any relationship to CWD, they need to have distinguishable characteristics."

John Pape (Disease Control and Environmental Epidemiology Division, Colorado Department of Public Health and Environment, Denver, CO, USA) told TLID that he and colleagues are taking a broad- brush look at deaths due to neurological diseases in Colorado. "We are examining the death certificates of all Colorado residents for a 32-year period." Pape hopes to get sufficient numbers to compare rates of death from various neurological disorders, including CJD, in CWD-endemic and non-endemic areas of Colorado.

"Until now findings have been reported on one case. NPDPSC pathologists have concluded that, although they __did not concur with the previous diagnosis of Pick's disease__,

a form of dementia, the autopsy tissue from 1993 showed no evidence of a prion-related disorder."

=========================

if CDC/NPDPSC says it's not CJD, NPDPSC/CDC says it's not Picks, then who do we call, ghostbusters???

what the heck is it then?

"For us to be able to tell whether these cases would have any relationship to CWD, they need to have distinguishable characteristics."

what if sCJD does _not_ have distinguishable characteristics, do we sit on our butts and just continue to say everything is o.k. it's all sporadic/spontaneous CJDs, even though we are getting more variants? plus, will these distinguishable characteristics look the same after lying around for 10 years? what if these distinguishable characteristics that they use to distinguish between various TSE have nothing to do with actually distinguishing anything other than a single TSE, but the titre of infectivity, the route, and the source is what plays the role in the different distinguishable characterisics they speak of?

'As implied in the Inset 25 we must not assume that transmission of BSE to other species will invariably present pathology typical of a scrapie-like disease.'

SEEMS Wisconsin is in the progress of making CJD TSE prion reportable by the document below ;

PRION DEMENTIA

Information for Wisconsin Medical Providers from the Wisconsin Division of Public Health and the Prion Disease Advisory Workgroup*

Introduction

The Wisconsin Division of Public Health (DPH) is in the _process_ of making human Transmissible Spongiform Encephalopathy (TSE) an officially reportable condition. Clinicians are strongly encouraged to report suspected TSE cases to the DPH. TSEs consist of any prion-related disease, including sporadic Creutzfeldt-Jakob Disease (sCJD), new variant Creutzfeldt-Jakob Disease (vCJD), Fatal Familial Insomnia and Gerstmann-Straüssler-Scheinker Syndrome. Of these, only sCJD is known to occur with any frequency in the US (incidence ~ 1 per million).

A workgroup consisting primarily of Wisconsin neurologists and neuropathologists was convened to develop the clinical criteria that should elicit a report of a possible TSE case. This expert panel has also developed guidelines to assist health care providers in the management /diagnosis of suspected prion-related dementias and has established a referral network consisting of clinicians with expertise in prion diseases. This document discusses these clinical guidelines and the referral network.

Criteria for Reporting a Suspect Case of TSE in Wisconsin

A prion-related disorder, such as Creutzfeldt-Jakob Disease (CJD), should be suspected and reported to the Wisconsin Division of Public Health in any patient with:

Dementia of early onset ( < 55 years) OR

Rapidly progressive dementia with one or more of the following:

· Movement disorders (e.g., myoclonus, ataxia)

· Painful sensory symptoms

· Visual disturbances

Diagnosis and Work-Up

It is the choice of the clinician whether to manage a patient with a suspected prion disease or whether to refer the patient to a specialty center or neurologist as discussed below. As in any patient with dementia, treatable causes should first be sought. Routine blood work would include a CBC, electrolytes, hepatic and renal function tests, B12 level and thyroid testing.

A careful neurological history and examination may reveal findings supportive of a diagnosis of a TSE, such as an insidious, nonspecific prodrome (fatigue, behavioral change, depression, weight loss, sleep disturbance), leading within months to relentless dementia, with prominent neurological signs such as myoclonus, ataxia, visual disturbance, corticospinal and extrapyramidal dysfunction, and akinetic mutism. Confirmatory diagnostic studies include:

· Cerebrospinal fluid: constituents are usually normal, except for elevated levels of the 14-3-3 neuron-specific enolase; sensitivity and specificity may approach 90%, however the usefulness of this test is questionable early in the disease. Research is ongoing to determine when in the course of the illness this becomes positive.

· Electroencephalogram: early in sCJD the EEG may be normal or may show non-specific slowing; later biphasic or triphasic synchronous complexes may be superimposed on a slow background; terminally, the EEG will show periodic sharp wave complexes in up to 90% of patients. These characteristic EEG findings are usually absent in cases of vCJD.

· Magnetic Resonance Imaging: CJD typically shows increased T2 and FLAIR signal in the basal ganglia and cerebral cortex in ~ 80% of patients at presentation. Diffusion-weighted MRI sequences are critical for optimal detection, with sensitivity and specificity approaching 100% in the appropriate clinical setting. For this reason, it is essential to order a diffusion-weighted-MRI study and consult with an experienced neuroradiologist when considering the diagnosis of CJD.

· Brain biopsy and Autopsy: Conventional pathological investigation will show spongiform encephalopathy and will allow submission of tissues to the national registry laboratory for specialized studies. We strongly recommend specimens be sent to the National Prion Disease Pathology Surveillance Center, Institute of Pathology, Case Western Reserve University, Cleveland, OH. Telephone: 216-368-0587, e-mail cjdsurv@po.cwru.edu. This Center can also perform genotyping on frozen specimens to determine the type of CJD and diagnose related conditions such as GSS and FFI. These services are offered free of charge.

· Infection Control Considerations – Autopsy and Embalming: World Health Organization (WHO) Infection Control Guidelines for Transmissible Spongiform Encephalopathies (TSE) should be followed during autopsy. An autopsied or To report a case, call Jim Kazmierczak, DVM, at the Division of Public Health at 608-266-2154 traumatized body of a suspected or confirmed TSE patient can be embalmed using the precautions outlined in the WHO TSE Infection Control Guidelines. www.who.int/emc-documents/tse/docs/whocdscsraph2003.pdf . Bodies that have not been autopsied or traumatized can be embalmed using Standard Precautions.

· Funding for Autopsies: Funds are available through the Division of Public Health to provide for transport for autopsy and / or autopsy costs of suspect TSE patients at a Wisconsin prion disease center. Please contact Dr, Jim Kazmierczak at 608-266-2154 for details.

New variant CJD (vCJD) was linked to beef consumption during the occurrence of a large outbreak of bovine spongiform encephalopathy (BSE, commonly known as mad cow disease) among cattle in the United Kingdom (UK) in the 1990’s. To date, despite an active USDA surveillance program, no case of this cattle disease has been identified in the US. The vCJD should not be confused with the classic form of sCJD that is endemic throughout the world. The median age at death of patients with sCJD is 68 years and very few cases occur in persons under 30 years of age. In contrast, the median age at death of patients with vCJD in the UK is 28 years. The vCJD can be confirmed only through examination of brain tissue. The incubation period for vCJD is unknown; however, it is likely that it will be measured in years or decades. In contrast to sCJD, vCJD in the UK predominantly affects younger people, has atypical clinical features, with prominent psychiatric or sensory symptoms at the time of clinical presentation and delayed onset of neurologic abnormalities, including ataxia within weeks or months, dementia and myoclonus late in the illness, a duration of illness of at least 6 months and a diffusely abnormal non-diagnostic EEG.

Further Resources: Wisconsin Prion Centers and Neurologists A network of medical centers and neurologists in Wisconsin has been established in order to assist health care professionals in the diagnosis, care, and reporting of possible human prion disease such as CJD. At each center, there are identified neurologists, neuro-radiologists, neuropathologists, and other professionals with expertise in prion diseases available for consultation or referral. If you would like to refer a patient to one of the centers, please call Jim Kazmierczak, at the Wisconsin Division of Public Health, 608-266-2154. Dr. Kazmierczak will fax this document and other pertinent information to the caller.

The goals of the network are to assist primary clinicians and neurologists in the accurate diagnosis of patients with suspected prion diseases, advise on the care of persons with prion diseases, promote public safety, enhance reporting to state health officials for surveillance purposes, and facilitate interactions with national prion research centers.

However, by this, CJD was made reportable April 8, 2008 in Wisonsin. I don't know why they have not updated the Government sites with this data ? Also, in the above official document from the Wisconsin DPH, they state, if this is true today, this is a total flaw in the surveillance program ;

A prion-related disorder, such as Creutzfeldt-Jakob Disease (CJD), should be suspected and reported to the Wisconsin Division of Public Health in any patient with: Dementia of early onset ( < 55 years ) OR...

BY only being concerned with CJD TSE prion victims of only less than 55 years old victims, you will continue to spread the agent via iatrogenic routes and sources. what do these officials think, that 55 years and older, that may or may not have clinical TSE prion disease, yet sub-clinically, they go on to have surgical, dental, donate blood, tissue, organs etc., that these 55 years and older will NEVER have a medical or surgical procedure? let me remind you that the very old can get nvCJD as well, if you are one of the fools that still believe in the UKBSEnvCJD only theory. ...and a fool you are if you still believe in the UKBSEnvCJD only theory. ...tss

(PRLEAP.COM) Alaska, Illinois and Wisconsin have all added Creutzfeldt-Jakob disease (CJD) to their required reportable conditions list. "This is wonderful to hear", remarked Christy Brom, the director/founder of CJD Aware!, a non-profit, information organization based in New Orleans, Louisiana. "State Health Departments are on the front-lines when dealing with public health issues, such as infectious diseases. By making CJD a reportable condition, health officials can gather data that shows how often the disease occurs, monitor the trends of the disease and continue their tracking of outbreaks." added Ms. Brom. All states have reportable conditions list, but requirements for reporting diseases varies from state-to-state.

Since its inception in the spring of 2002, CJD Aware! continues to grow at a steady pace. The information organization has nearly tripled its database of individuals, medical professionals and educators who have contacted the organization requesting information about Creutzfeldt-Jakob disease (CJD). In addition to monitoring several state Health Departments as some revise their rules/regulations, CJD Aware! is also continuing their annual ‘CJD Awareness Week’ campaign.

"This campaign will go on until we have proclamations from the governors of all 50 states," remarked Sandy Rouse, a CJD Aware! volunteer. "We have volunteers ready to assist us this year with Virginia, Oklahoma, South Carolina and Florida," added Ms. Rouse. CJD Aware! has seen a surge in requests for their information packets, as well as a recent request from a dementia clinic for all their available information.

As CJD Aware! begins a very busy and growth-oriented time, they find it fitting that this year, 2008, is the 25th anniversary of the Orphan Drug Act. Signed on Janury 4, 1983 by President Ronald Reagan, this legislation brings new hope to the 25 million Americans who suffer from rare diseases. The staff and volunteers continue with CJD Aware’s! mission of "sharing information to find a cure" in each packet that is mailed out, each email that is responded to and each phone call that is answered. "In our 6 years of existence, it is extremely gratifying for CJD Aware! to feel that we have helped families understand and deal with this very rare and devastating disease" says Ms. Brom. Contact Information Christy Brom CJD Aware!

MADISON, Wis. -- University of Wisconsin Hospital officials said Thursday that at least 53 patients were potentially exposed to a rare neurological disease. A woman in her 50s died of classic Creutzfeldt-Jakob disease Tuesday. She underwent surgery at UW Hospital on June 11, WISC-TV reported. The disease was diagnosed Monday, after a brain biopsy. The state Department of Health Services said the patient died from the classic case of Creutzfeldt-Jakob disease, a naturally occurring but very rare neurological disorder. UW Hospital officials said they believe there is a small chance that 53 other patients there were exposed to the disease, but the hospital has alerted those involved. Health officials said there is not a public health threat beyond the 53 people. Those patients underwent surgeries or procedures where instruments used on the CJD patient were possibly used during their procedures, either on brain tissue or spinal tissue. So far, none of the 53 people have shown any symptoms of the neurological disorder, but the disease can take years to show symptoms, WISC-TV reported. Clinical signs and symptoms of classic CJD include dementia and early neurologic signs. The median age of those with CJD is 68.

Since its introduction into the diagnostic criteria for sporadic CJD in 1998, the analysis of cerebrospinal fluid (CSF) for 14-3-3 has become a widely accepted investigation in patients with suspected sporadic CJD. However, a number of reports have raised concerns about its lack of specificity. This has prompted the search for a more specific and disease-related pre-mortem diagnostic test for sporadic CJD. The ability of PrPSc to convert PrPC into protease-resistance isoforms has been exploited using a variety of techniques such as protein misfolding cyclic amplification (PMCA) and quaking induced conversion (QuIC). A recent adaptation of QuIC (real-time QuIC) has been described which incorporates thioflavin T (ThT) in the reaction mixture. The ThT binds to the aggregated PrP causing a change in the ThT emission spectrum that can be monitored in real-time. Recent studies have shown that CSF samples from hamsters inoculated with experimental scrapie, sheep with scrapie and patients with sporadic CJD can be correctly identified using real-time QuIC.1,2 We now describe the findings of an investigation into the value of real-time QuIC in the diagnosis of sCJD. A blinded panel of CSF samples from 56 neuropathologically confirmed cases of sCJD and from 53 patients who were initially suspected of having sCJD but who were found to have an alternative diagnosis were analyzed. Of the 56 patients with sCJD 51 were found to give a positive response with real-time QuIC. In contrast only one patient from the control group was found to be positive. The sensitivity and specificity was 91% and 98%, respectively. The corresponding sensitivity and specificity of CSF 14-3-3 was 91% and 55%, respectively. These results suggest that real-time QuIC has the potential to be a more specific pre-mortem CSF test for sCJD than CSF 14-3-3.

HOW RELIABLE IS REVIEWING DEATH CERTIFICATE DATA FOR SURVIELLANCE OF CJD, instead of making CJD and all Prion disease reportable Nationally ???

it's not reliable at all, that's the whole purpose of only _reviewing_ death certificate data for CJD TSE prion disease, and not making it reportable Nationally, Government officials do NOT want the public to know how many cases there are. IT's the same as with mad cow disease, if you don't look, you don't find. simple as that. ...tss

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will

snip...

IDENTIFICATION OF CASES

Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

The constant increase of chronic wasting disease (CWD) incidence in North America raises a question about their zoonotic potential. A recent publication showed their transmissibility to new-world monkeys, but no transmission to old-world monkeys, which are phylogenetically closer to humans, has so far been reported. Moreover, several studies have failed to transmit CWD to transgenic mice overexpressing human PrP.

Bovine spongiform encephalopathy (BSE) is the only animal prion disease for which a zoonotic potential has been proven. We described the transmission of the atypical BSE-L strain of BSE to cynomolgus monkeys, suggesting a weak cattle-to-primate species barrier. We observed the same phenomenon with a cattle-adapted strain of TME (Transmissible Mink Encephalopathy). Since cattle experimentally exposed to CWD strains have also developed spongiform encephalopathies, we inoculated brain tissue from CWD-infected cattle to three cynomolgus macaques as well as to transgenic mice overexpressing bovine or human PrP. Since CWD prion strains are highly lymphotropic, suggesting an adaptation of these agents after peripheral exposure, a parallel set of four monkeys was inoculated with CWD-infected cervid brains using the oral route.

Nearly four years post-exposure, monkeys exposed to CWD-related prion strains remain asymptomatic. In contrast, bovinized and humanized transgenic mice showed signs of infection, suggesting that CWD-related prion strains may be capable of crossing the cattle-to-primate species barrier. Comparisons with transmission results and incubation periods obtained after exposure to other cattle prion strains (c-BSE, BSE-L, BSE-H and cattle-adapted TME) will also be presented, in order to evaluate the respective risks of each strain.

Thank you for your recent letter concerning the publication of the third annual report from the CJD Surveillance Unit. I am sorry that you are dissatisfied with the way in which this report was published.

The Surveillance Unit is a completely independant outside body and the Department of Health is committed to publishing their reports as soon as they become available. In the circumstances it is not the practice to circulate the report for comment since the findings of the report would not be amended. In future we can ensure that the British Deer Farmers Association receives a copy of the report in advance of publication.

The Chief Medical Officer has undertaken to keep the public fully informed of the results of any research in respect of CJD. This report was entirely the work of the unit and was produced completely independantly of the the Department.

The statistical results reqarding the consumption of venison was put into perspective in the body of the report and was not mentioned at all in the press release. Media attention regarding this report was low key but gave a realistic presentation of the statistical findings of the Unit. This approach to publication was successful in that consumption of venison was highlighted only once by the media ie. in the News at one television proqramme.

I believe that a further statement about the report, or indeed statistical links between CJD and consumption of venison, would increase, and quite possibly give damaging credence, to the whole issue. From the low key media reports of which I am aware it seems unlikely that venison consumption will suffer adversely, if at all.

In the Archives of Neurology you quoted (the abstract of which was attached to your email), we did not say CWD in humans will present like variant CJD.

That assumption would be wrong. I encourage you to read the whole article and call me if you have questions or need more clarification (phone: 404-639-3091). Also, we do not claim that "no-one has ever been infected with prion disease from eating venison." Our conclusion stating that we found no strong evidence of CWD transmission to humans in the article you quoted or in any other forum is limited to the patients we investigated.

We intracerebrally challenged four species of native North American rodents that inhabit locations undergoing cervid chronic wasting disease (CWD) epidemics. The species were: deer mice (Peromyscus maniculatus), white-footed mice (P. leucopus), meadow voles (Microtus pennsylvanicus), and red-backed voles (Myodes gapperi). The inocula were prepared from the brains of hunter-harvested white-tailed deer from Wisconsin that tested positive for CWD. Meadow voles proved to be most susceptible, with a median incubation period of 272 days. Immunoblotting and immunohistochemistry confirmed the presence of PrPd in the brains of all challenged meadow voles. Subsequent passages in meadow voles lead to a significant reduction in incubation period. The disease progression in red-backed voles, which are very closely related to the European bank vole (M. glareolus) which have been demonstrated to be sensitive to a number of TSEs, was slower than in meadow voles with a median incubation period of 351 days. We sequenced the meadow vole and red-backed vole Prnp genes and found three amino acid (AA) differences outside of the signal and GPI anchor sequences. Of these differences (T56-, G90S, S170N; read-backed vole:meadow vole), S170N is particularly intriguing due its postulated involvement in “rigid loop” structure and CWD susceptibility. Deer mice did not exhibit disease signs until nearly 1.5 years post-inoculation, but appear to be exhibiting a high degree of disease penetrance. White-footed mice have an even longer incubation period but are also showing high penetrance. Second passage experiments show significant shortening of incubation periods. Meadow voles in particular appear to be interesting lab models for CWD. These rodents scavenge carrion, and are an important food source for many predator species. Furthermore, these rodents enter human and domestic livestock food chains by accidental inclusion in grain and forage. Further investigation of these species as potential hosts, bridge species, and reservoirs of CWD is required.

Now recognized in 18 states in the US, two Canadian provinces, and one Asian country, efficient horizontal transmission is a signature trait of chronic wasting disease (CWD) of cervids. The facile spread of CWD appears linked to the prion/host relationship facilitating efficient mucosal uptake, peripheral lymphoreticular amplification, and horizontal dissemination exploiting excretory tissues and their products. In addition, recent studies suggest the likelihood of early life mother to offspring transmission. Growing evidence from studies of cervid CWD exposure by natural routes indicate that the incubation period for overt infection detection and disease onset (if any) may be much longer than originally thought.

Whether non-cervid species (including humans) may be susceptible to CWD infection and/or act as reservoirs for infection in nature remains unknown. In vitro and in vivo studies of the CWD species barrier indicate the potential for a host range extending beyond cervid species, although no evidence for this has thus far been detected in nature. Interestingly, rodent and mustelid species sympatric with free ranging cervids have been shown susceptible to CWD prions and such trans-species infection broadens the host range/strain characteristics of CWD prions. While the origins of CWD remain unknown, the relationship between sheep scrapie and CWD and the existence of multiple CWD prion strains/quasispecies remain interesting and merit further investigation.

Purpose: While the exact mechanisms of chronic wasting disease (CWD) prion transmission, entry, and trafficking remain incompletely elucidated, transmission by exposure of the oral and/or nasal mucous membranes seems certain. As part of foraging, cervids likely experience minor lesions in the oral mucous membranes; these could have impact on susceptibility to prion entry and subsequent infection. To explore this potential co-factor, we used cervid PrP transgenic mice to assess whether or not micro-abrasions to the tongue may enhance susceptibility to oral CWD infection and whether or not infectious CWD PrPCWD could be detected immediately after exposure.

Methods: Two sets of FVB mice transgenically expressing the normal cervid PrPC protein [Tg(CerPrP-E226)5037+/-], with or without abrasions on the lingual mucosa, were inoculated orally with 10µl of a 10% w/v brain homogenate from either CWD-positive or negative deer. Abrasions were created by lightly scratching the dorsal lingual epithelium with a 27g needle. Cohorts were sacrificed at either early [0, 1, and 4 h post inoculation (pi)] or late [3, 12, and 24 months pi] time points or when signs of neurologic disease were observed. Tongue, lymphoid tissue, and the brain were assessed by western blotting and tyramide signal amplification (TSA) immunohistochemistry to detect the CWD abnormal prion protein (PrPCWD).

Results: Between 296 and 515 dpi, 9 of the 9 CWD-inoculated mice with lingual lesions developed clinical signs of neurologic dysfunction mandating euthanasia. Only the brain in all nine mice was positive for PrPCWD by western blot and TSA immunohistochemistry. Conversely, all mice without oral lesions remained asymptomatic for >700 dpi and no evidence of PrPCWD was detected in these mice terminally. Moreover, no evidence of PrPCWD could be detected when the micro-abrasion sites were examined at 0, 1, or 4 h after oral exposure or at any pre-terminal time point thereafter.

Conclusions: Micro-abrasions to the lingual surface substantially facilitated CWD transmission, suggesting that minor oral mucosal lesions may be a significant co-factor facilitating infection in foraging cervids or other species.

Oral.27: Identification of PrPCWD in the Salivary Gland Epithelium of White-Tailed Deer: Novel Insights Into Mechanisms of CWD Horizontal Transmission

Background. Chronic wasting disease (CWD) of cervids is characterized by its efficient transmission among animals. Although bioassay and in vitro amplification studies have confirmed the infectious nature of saliva, urine, blood and feces, uncertainties remain regarding the mechanisms of this facile horizontal transmission. Notable among these is a specific understanding of the means by which prion infectivity is transferred to a body fluid or excretion.

Objectives. The chief objective of this work was to provide tissue-level insights into the process of prion shedding via the salivary glands by means of enhanced immunohistochemistry (IHC).

Results. Here we show that enhanced IHC techniques are capable of detecting pathogenic prion protein (PrPCWD) in the salivary glands of infected WTD. Utilizing optimized TSA we have detected granular to clumped, intra-cytoplasmic PrPCWD deposits in parotid and mandibular salivary gland ductular epithelial cells of WTD infected with CWD for 19 to 27 months. Salivary PrPCWD was not detected in sham-inoculated or naïve WTD. PrPCWD was not identified in any other salivary gland cell types.

Discussion. We present immunohistochemical evidence for PrPCWD accumulation in the salivary gland ductules, which provides a tissue level correlate to the infectivity present in cervid saliva and may explain the manner by which prions transit to saliva, and thereby facilitate the high degree of CWD horizontal transmission. These findings complement work by Haley et al. (this symposium) demonstrating the presence of CWD prions in salivary glands through the in vitro amplification assay PMCA. 12 Prion Volume 5 Supplement

Infectious diseases are increasingly recognized as an important force driving population dynamics, conservation biology and natural selection in wildlife populations. Infectious agents have been implicated in the decline of small or endangered populations and may act to constrain population size, distribution, or growth rates. Further, diseases may provide selective pressures that shape the genetic diversity of populations or species. Thus understanding disease dynamics and selective pressures from pathogens is crucial to understanding population processes, managing wildlife diseases, and conserving biological diversity. There is ample evidence that variation in the PRNP gene impacts host susceptibility to TSEs. Research in human TSEs and scrapie, as well as chronic wasting disease (CWD) has demonstrated that PRNP variation influences the susceptibility and progression of prion diseases. Still, little is known about how these genetic differences might influence natural selection within cervid populations or how genetic make-up might shape disease dynamics and the population response to CWD. Here we determine the links between genetic variation, CWD transmission, and susceptibility of white-tailed deer (Odocoileus virginianus) with implications for fitness and disease-driven genetic selection. We developed a single nucleotide polymorphism (SNP) assay to efficiently genotype deer at the 96th codon of the PRNP gene. We then used a Bayesian modeling approach to calculate genotype-specific infection and disease mortality rates. We found that the more susceptible (homozygous) genotype had over four times greater risk of CWD infection and, once infected, deer with the resistant (heterozygous) genotype survived 49% longer (8.25 more months). We used these epidemiological parameters in a multi-stage population matrix model to evaluate genotype-specific population growth. The differences in disease infection and mortality rates allowed genetically resistant deer to achieve higher population growth and obtain a long-term fitness advantage. This differential fitness produced a selection coefficient of over 1% favoring the heterozygous CWD-resistant genotype. Such strong selective pressure suggests that the resistant allele could become dominant in the population within just a few hundred years, extremely rapid on the evolutionary time scale. Our results have direct implications for the epidemiology, dynamics, and future trends in CWD transmission and spread.

Domestic and non-domestic cats have been shown to be susceptible to one prion disease, feline spongiform encephalopathy (FSE), thought to be transmitted through consumption of bovine spongiform encephalopathy (BSE) contaminated meat. Because domestic and free ranging felids scavenge cervid carcasses, including those in CWD affected areas, we evaluated the susceptibility of domestic cats to CWD infection experimentally. Groups of n = 5 cats each were inoculated either intracerebrally (IC) or orally (PO) with CWD deer brain homogenate. Between 40–43 months following IC inoculation, two cats developed mild but progressive symptoms including weight loss, anorexia, polydipsia, patterned motor behaviors and ataxia—ultimately mandating euthanasia. Magnetic resonance imaging (MRI) on the brain of one of these animals (vs. two age-matched controls) performed just before euthanasia revealed increased ventricular system volume, more prominent sulci, and T2 hyperintensity deep in the white matter of the frontal hemisphere and in cortical grey distributed through the brain, likely representing inflammation or gliosis. PrPRES and widely distributed peri-neuronal vacuoles were demonstrated in the brains of both animals by immunodetection assays. No clinical signs of TSE have been detected in the remaining primary passage cats after 80 months pi. Feline-adapted CWD was sub-passaged into groups (n=4 or 5) of cats by IC, PO, and IP/SQ routes. Currently, at 22 months pi, all five IC inoculated cats are demonstrating abnormal behavior including increasing aggressiveness, pacing, and hyper responsiveness. Two of these cats have developed rear limb ataxia. Although the limited data from this ongoing study must be considered preliminary, they raise the potential for cervid-to-feline transmission in nature.

Oral.44: Genetic Variability and Association with Prion Disease Susceptibility of the Prion Gene in the Mammalian Order Carnivora

Carnivores are exposed to significant levels of CWD in some regions of the US and Canada. Indeed it has been proposed recently that mountain lions prey selectively on prion–infected mule deer. It is likely that predators have also at least occasionally been exposed to other prion diseases, such as sheep scrapie in other countries. How susceptible are predators and scavengers to prion diseases? It is well known that the prion protein sequence is important as a major modulator of susceptibility and pathogenesis of prion disease. For example, prion disease susceptibility in sheep, goats and deer is modulated by at least 15 different polymorphisms of the PrP protein. PrP sequencing of carnivore species has not been done in great numbers and the degree of genetic variation of their PrP in wild and domesticated populations has not been addressed in any detail. However, to estimate the genetic risk of populations to diseases such as CWD one needs to understand the genetic variation of the target species.

We have analyzed the prion protein sequence of over 450 samples from over 20 species/subspecies of the suborders feliformia (cat-like) and caniformia (dog-like) representing ~320 samples from wild populations (US, Europe), ~110 samples from companion animals and ~25 samples from zoo collections. Within these samples were nine FSE cat cases, including the index case from the UK and six FSE cheetahs.

We established the PrP protein variants in our sample set and conclude that the number of PrP variants is small, with slightly more variability in caniformia than feliformia. All feline prion sequences have a characteristic alanine change in their repeat region that is not seen in any other species; all canine PrP encode aspartic acid in position 163, which is not present in any other species with the exception of wolverines. We hypothesis that these differences may explain some of the difference observed in prion disease susceptibility. The analysis of the FSE cases revealed no additional mutations therefore excluding the possibility of particularly susceptible PrP genotypes.

Although the general susceptibility of predators to CWD has not been established, we predict that it is unlikely that species

such as mountain lion and black bear will be protected by resistant alleles, whereas wolf and wolverine may have a slightly higher susceptibility threshold.

W.G. and P.S. supported by Institute Strategic Grant funding from the BBSRC, UK.

Many Indigenous communities in western Canada are concerned about increases in wildlife disease and environmental contamination that threaten their longstanding use of moose, elk and deer. Over the last three years we have been exploring the implications of these changes in close collaboration with two First Nations in Alberta (Alexis Nakota Sioux Nation and Paul First Nation) and a third First Nation in Saskatchewan (Cote First Nation). The overall goal of our project has been to better understand and respond to chronic wasting disease and other potential threats to wildlife and environmental health that confront these communities. The project is holistic and cross-cultural in approach, bridging Western science with Traditional Knowledge, and has been shaped and controlled by the community partners at all stages.

The objective of the first phase of the project was to better understand the implications of CWD as well as the role of cervids (i.e. moose, elk, and deer) in the diets and culture of these communities. It focused on interviews and participatory mapping. The objective of the second phase was to identify any factors responsible for ongoing declines in the health of cervids, and to assess to what degree these declines have been associated with CWD and the adverse effects of surrounding oil and gas extraction, agriculture and deforestation. Hunters collected samples of moose, deer, and elk and these samples have been tested for CWD, parasites, heavy metals, and other contaminants.

The objective of third phase was to explore the role of risk communication regarding CWD as it affects Indigenous communities and other stakeholders. This was, in part, achieved by evaluating media coverage and governmental communication with Indigenous communities regarding CWD and more generally wildlife health. Results show there has been little effective communication with or inclusion of Indigenous Peoples in government risk communication strategies. What communication did exist was generally culturally inappropriate, inaccessible, and, often generated community concern and even fear. However, we have developed a number of effective approaches to knowledge exchange in this collaborative project. They include an interactive website (www.inlandandlife.ca), plain-language newsletters, participatory video, and campouts on the land.

Outcomes of all three phases of this project have important implications for the understanding and communication of risks associated with CWD as they affect Indigenous communities. Yet, our findings are also relevant for risk communication with many stakeholders and a wide variety of threats associated with declining wildlife and environmental health.

Introduction: Chronic wasting disease (CWD) has been occurring for several decades among wild cervids in Colorado and Wyoming. The increasing detection of CWD in an additional 12 US states and two Canadian provinces may have resulted in increased human exposure to CWD. Although studies have evaluated the possible transmission of CWD to humans in laboratory models, a reliable assessment requires conducting epidemiologic and laboratory studies designed to identify prion disease among humans exposed to CWD and generating scientific evidence causally linking the two illnesses.

Methods: In collaboration with the Centers for Disease Control and Prevention, the Wyoming Department of Health and the Colorado Department of Public Health and Environment established a long-term follow-up study of hunter data to monitor the potential CWD transmission to humans. Personal identifiers from deer or elk hunter database are cross-checked with mortality data to determine their mortality status and causes of death.

Results: In Colorado, the hunter data include about 4.9 million records of licenses purchased during 1995–2008, representing about 1.1 million hunters. Overall, 48% of hunters purchased a license to hunt in areas that included CWD positive game units and 47% to hunt anywhere in Colorado. In Wyoming, the data include about 1.2 million records of licenses purchased during 1996-2009, representing about 0.5 million hunters; 34% of hunters purchased a license to hunt in areas that included CWD positive game units and 28% to hunt anywhere in Wyoming. During the study period three Colorado hunters (expected number: 3-15 cases) and three Wyoming hunters (expected number: 0-6 cases) were identified to have died of Creutzfeldt-Jakob disease (CJD).

Conclusions: No evidence suggests that the CJD incidence is higher than expected among persons who hunted in Colorado or Wyoming. The hunter data are valuable for monitoring the potential transmission of CWD to humans. Ongoing assessment and long-term follow-up of the hunter population is necessary because human prion diseases are associated with long latency periods and the pathogenicity of CWD might change over time.

Prion strains have been identified in virtually every species affected by these transmissible neurological disorders. Prion strains can exhibit unique clinical symptoms, disease incubation period, biochemical characteristics of the prion protein, as well as species tropism. We have previously demonstrated that specific PRNP polymorphisms are linked to resistance to chronic wasting disease (CWD) infection in free-ranging white-tailed deer populations. In hunter-harvested CWD-positive deer, the "wild-type" alleles (with glutamine at 95 aa and glycine at 96 aa) were over-represented while the 95 (95H) and 96 (96S) polymorphisms were under-represented. Experimental oral infection of white-tailed deer with known PRNP genotypes (with inoculum from CWD-positive wt/wt deer) confirmed this link between prion protein primary sequence and incubation period. All orally infected animals became clinically positive for CWD. The wt/wt had the shortest incubation period (693 dpi); the animal heterozygous for 95H/96S the longest (1596 dpi). Analysis of the CWD isolates revealed biochemical and biophysical differences between PrPCWD from wt/wt deer and H95/S96 deer. These proteins can be distinguished by full-length PrP glycoform patterns, proteinase K resistance, molecular weight, sedimentation properties in N-Lauroylsarkosine and structural stability to chaotropes. The presence of these PrPCWD isoforms suggests that white-tailed deer can generate several different strains of CWD agent; potentially with different transmission properties.

DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

-------- Original Message --------

Subject: DOCKET-- 03D-0186 -- FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

Date: Fri, 16 May 2003 11:47:37 -0500

From: "Terry S. Singeltary Sr."

To: fdadockets@oc.fda.gov

Greetings FDA,

i would kindly like to comment on;

Docket 03D-0186

FDA Issues Draft Guidance on Use of Material From Deer and Elk in Animal Feed; Availability

Several factors on this apparent voluntary proposal disturbs me greatly, please allow me to point them out;

1. MY first point is the failure of the partial ruminant-to-ruminant feed ban of 8/4/97. this partial and voluntary feed ban of some ruminant materials being fed back to cattle is terribly flawed. without the _total_ and _mandatory_ ban of all ruminant materials being fed back to ruminants including cattle, sheep, goat, deer, elk and mink, chickens, fish (all farmed animals for human/animal consumption), this half ass measure will fail terribly, as in the past decades...

2. WHAT about sub-clinical TSE in deer and elk? with the recent findings of deer fawns being infected with CWD, how many could possibly be sub-clinically infected. until we have a rapid TSE test to assure us that all deer/elk are free of disease (clinical and sub-clinical), we must ban not only documented CWD infected deer/elk, but healthy ones as well. it this is not done, they system will fail...

3. WE must ban not only CNS (SRMs specified risk materials), but ALL tissues. recent new and old findings support infectivity in the rump or ass muscle. wether it be low or high, accumulation will play a crucial role in TSEs.

4. THERE are and have been for some time many TSEs in the USA. TME in mink, Scrapie in Sheep and Goats, and unidentified TSE in USA cattle. all this has been proven, but the TSE in USA cattle has been totally ignored for decades. i will document this data below in my references.

5. UNTIL we ban all ruminant by-products from being fed back to ALL ruminants, until we rapid TSE test (not only deer/elk) but cattle in sufficient numbers to find (1 million rapid TSE test in USA cattle annually for 5 years), any partial measures such as the ones proposed while ignoring sub-clinical TSEs and not rapid TSE testing cattle, not closing down feed mills that continue to violate the FDA's BSE feed regulation (21 CFR 589.2000) and not making freely available those violations, will only continue to spread these TSE mad cow agents in the USA. I am curious what we will call a phenotype in a species that is mixed with who knows how many strains of scrapie, who knows what strain or how many strains of TSE in USA cattle, and the CWD in deer and elk (no telling how many strains there), but all of this has been rendered for animal feeds in the USA for decades. it will get interesting once someone starts looking in all species, including humans here in the USA, but this has yet to happen...

6. IT is paramount that CJD be made reportable in every state (especially ''sporadic'' cjd), and that a CJD Questionnaire must be issued to every family of a victim of TSE. only checking death certificates will not be sufficient. this has been proven as well (see below HISTORY OF CJD -- CJD QUESTIONNAIRE)

7. WE must learn from our past mistakes, not continue to make the same mistakes...

Mule deer fawns (Odocoileus hemionus) were inoculated orally with a brain homogenate prepared from mule deer with naturally occurring chronic wasting disease (CWD), a prion-induced transmissible spongiform encephalopathy. Fawns were necropsied and examined for PrP res, the abnormal prion protein isoform, at 10, 42, 53, 77, 78 and 80 days post-inoculation (p.i.) using an immunohistochemistry assay modified to enhance sensitivity. PrPres was detected in alimentary-tract-associated lymphoid tissues (one or more of the following: retropharyngeal lymph node, tonsil, Peyer's patch and ileocaecal lymph node) as early as 42 days p.i. and in all fawns examined thereafter (53 to 80 days p.i.). No PrPres staining was detected in lymphoid tissue of three control fawns receiving a control brain inoculum, nor was PrPres detectable in neural tissue of any fawn. PrPres-specific staining was markedly enhanced by sequential tissue treatment with formic acid, proteinase K and hydrated autoclaving prior to immunohistochemical staining with monoclonal antibody F89/160.1.5. These results indicate that CWD PrP res can be detected in lymphoid tissues draining the alimentary tract within a few weeks after oral exposure to infectious prions and may reflect the initial pathway of CWD infection in deer. The rapid infection of deer fawns following exposure by the most plausible natural route is consistent with the efficient horizontal transmission of CWD in nature and enables accelerated studies of transmission and pathogenesis in the native species.

snip...

These results indicate that mule deer fawns develop detectable PrP res after oral exposure to an inoculum containing CWD prions. In the earliest post-exposure period, CWD PrPres was traced to the lymphoid tissues draining the oral and intestinal mucosa (i.e. the retropharyngeal lymph nodes, tonsil, ileal Peyer's patches and ileocaecal lymph nodes), which probably received the highest initial exposure to the inoculum. Hadlow et al. (1982) demonstrated scrapie agent in the tonsil, retropharyngeal and mesenteric lymph nodes, ileum and spleen in a 10-month-old naturally infected lamb by mouse bioassay. Eight of nine sheep had infectivity in the retropharyngeal lymph node. He concluded that the tissue distribution suggested primary infection via the gastrointestinal tract. The tissue distribution of PrPres in the early stages of infection in the fawns is strikingly similar to that seen in naturally infected sheep with scrapie. These findings support oral exposure as a natural route of CWD infection in deer and support oral inoculation as a reasonable exposure route for experimental studies of CWD.

Abstract — Chronic wasting disease (CWD), an important emerging prion disease of cervids, is readily transmitted by intracerebral or oral inoculation from deer-to-deer and elk-to-elk, suggesting the latter is a natural route of exposure. Studies of host range susceptibility to oral infection, particularly of those species found in habitats where CWD currently exists are imperative. This report describes the experimental transmission of CWD to red deer following oral inoculation with infectious CWD material of elk origin. At 18 to 20 months post-inoculation, mild to moderate neurological signs and weight loss were observed and animals were euthanized and tested using 3 conventional immunological assays. The data indicate that red deer are susceptible to oral challenge and that tissues currently used for CWD diagnosis show strong abnormal prion (PrPCWD) accumulation. Widespread peripheral PrPCWD deposition involves lymphoreticular tissues, endocrine tissues, and cardiac muscle and suggests a potential source of prion infectivity, a means of horizontal transmission and carrier state.

SNIP...

There is a strong correlation between the presence of PrPTSE and infectivity in prion diseases. Although the epidemiologic evidence strongly suggests that CWD is not transmissible to humans, this study and others suggest caution in this regard. The finding of PrPCWD in various organs, albeit in clinical CWD, suggests that humans who consume or handle meat from CWD-infected red deer may be at risk of exposure to CWD prions. This study found that red deer tissues other than nervous and lymphoid tissue can support CWD prion replication and accumulation. As a result, the consumption or handling of meat from CWD-infected red deer will put humans at risk of exposure to CWD prions. In spite of a well-documented species barrier, a cautious approach would involve preventing such tissues from entering the animal and human food chains. Future studies will require sensitive and quantitative techniques such as bioassays in transgenic mice that assess tissue infectivity and quantitative immunoassays adapted to PrPCWD detection in peripheral tissues.

SNIP...

The exact mode of transmission of CWD in nature remains unclear but is believed to involve direct animal-to-animal contact or environmental contamination. As TSE agents are extremely resistant in the environment (39), oral exposure is the most plausible pathway by which the CWD prion may be introduced to deer in nature and represents a significant obstacle to eradication of CWD from either farmed or free-ranging cervid populations. The distribution of PrPCWD in gut-associated lymphoid tissues, salivary glands, and nasal mucosa in the red deer of this study suggests potential routes of PrPCWD shedding into the environment via fluids such as saliva or feces. However, this study did not identify the point at which an animal may become infectious during the course of infection. An improved understanding of the mechanisms of shedding and transmission will be important in the future management of CWD.

SNIP...

In summary, this study demonstrates the potential for oral transmission of CWD to red deer and describes the pattern of PrPCWD accumulation for this species. The current surveillance testing regime for cervids would be expected to identify CWD-infected red deer should it occur in North America. These results confirm the usefulness of rapid tests such as ELISA but with generally slightly lower sensitivity when compared with IHC when testing tissues with patchy or sporadic PrPCWD deposition. The finding of PrPCWD in several extraneural tissues including cardiac muscle and the endocrine system suggests that further investigation and monitoring of the potential transmissibility to other species including humans is warranted.

Atypical Scrapie Isolates Involve a Uniform Prion Species with a Complex Molecular Signature

PrPres peptides of low molecular mass have also been described in other types of prion disease, such as Gerstmann-Sträussler-Scheinker disease [32], [33] and Creutzfeldt-Jakob disease [34], [35] in humans as well as in H-BSE in cattle [36], [37]. Forthcoming directions of research are likely to focus on more precise comparative analyses of truncated PrPres peptides and their role in the biology of human and animal prion diseases...

Although prion diseases, such as Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the “scrapie form” (PrPSc), are used as disease markers, and for taxonomic purposes, in correlation with clinical, pathological, and genetic data. In humans, prion diseases can arise sporadically (sCJD) or genetically (gCJD and others), caused by mutations in the PrP-gene (PRNP), or as a foodborne infection, with the agent of bovine spongiform encephalopathy (BSE) causing variant CJD (vCJD). Person-to-person spread of human prion disease has only been known to occur following cannibalism (kuru disease in Papua New Guinea) or through medical or surgical treatment (iatrogenic CJD, iCJD). In contrast, scrapie in small ruminants and chronic wasting disease (CWD) in cervids behave as infectious diseases within these species. Recently, however, so-called atypical forms of prion diseases have been discovered in sheep (atypical/Nor98 scrapie) and in cattle, BSE-H and BSE-L. These maladies resemble sporadic or genetic human prion diseases and might be their animal equivalents. This hypothesis also raises the significant public health question of possible epidemiological links between these diseases and their counterparts in humans.

A distinct strain of scrapic identified in sheep of Norway in 1998 has since been identified in numerous countries throughout Europe. The disease is known as Nor98 or Not-98-like scrapic. among other names. Distinctions between classic scrapie and Nor98 scrapie are made based on histopathologv and immunodiagnostic results. There are also differences in the epidemiology, typical signalment, and likelihood of clinical signs being observed. In addition, sheep that have genotypes associated with resistance to classic scrapie are not spared from Nor98 disease. The various differences between classic and Nor98 scrapie have been consistently reported in the vast majority of cases described across Europe. The current study describes in detail the patholo gic changes and diagnostic results of the first 6 cases of' Nor98 scrapic disease diagnosed in sheep of the United States.

The first case identified as consistent with Nor98 scrapie had nonclassic PrP distribution in brain tissue, no PrPSC in lymph tissue, and nonclassic migration of protein bands on a Western blot test. The animal was an aged, mottled-faced ewe that was traced back to a commercial flock in Wyoming. ...

Case 2

The second case was a clinically normal 8-year-old Suffolk ewe that had been in a quarantined flock for 5 years at a USDA facility in Iowa.

Case 3

A 16-year-old, white-faced, cross-bred wether was born to a black-faced ewe. He lived his entire life as a pet on a farm in California.

Case 4

The fourth case of Nor98 scrapie was identified in an approximately 8-year-old Dorset ewe that was born into a flock of approximately 20 ewes in Indiana.

Case 5

The fifth case was a clinically normal, approximately 3-year-old, white-faced, cross-bred ewe from an approximately 400 head commercial flock in Minnesota.

Case 6

The sixth case of Nor98 scrapie was identified in a 4-year-old, white-faced ewe that was purchased and added to a commercial flock in Pennsylvania

This is an interesting editorial about the Mad Cow Disease debacle, and it's ramifications that will continue to play out for decades to come ;

Monday, October 10, 2011

EFSA Journal 2011 The European Response to BSE: A Success Story

snip...

EFSA and the European Centre for Disease Prevention and Control (ECDC) recently delivered a scientific opinion on any possible epidemiological or molecular association between TSEs in animals and humans (EFSA Panel on Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical BSE prions as the only TSE agents demonstrated to be zoonotic so far but the possibility that a small proportion of human cases so far classified as "sporadic" CJD are of zoonotic origin could not be excluded. Moreover, transmission experiments to non-human primates suggest that some TSE agents in addition to Classical BSE prions in cattle (namely L-type Atypical BSE, Classical BSE in sheep, transmissible mink encephalopathy (TME) and chronic wasting disease (CWD) agents) might have zoonotic potential.

Ongoing review of clinical and pathologic records of CJD decedents aged <55 years

Death certificate data review is effective as a surveillance tool for CJD:

SNIP...

Hunter study Goal: To determine whether chronic wasting disease (CWD), a prion disease of deer and elk, can cause disease in humans ƒáStudy: Follow-up of persons who hunted in Colorado and Wyoming, where CWD is found, and identifying those who died of prion disease

Results: Prion disease cases among this group within expected range so far

SNIP...

The lower incidence of CJD among Hispanics in the US may be at least partly due to: Underreporting of Hispanic ethnicity on death certificates relative to surveys and censuses.

SNIP...

Conclusion Collaboration with medical and public health personnel, NPDPSC, the CJD Foundation, and CDC is essential.

"Conclusion Collaboration with medical and public health personnel, NPDPSC, the CJD Foundation, and CDC is essential."

yada, yada, yada...same old song and dance $$$

YEP, it's ESSENTIAL for one thing, and one thing only, keeping all human TSE prion disease in the USA 'SPORADIC' CJD or 'SPORADIC' HUMAN TSE OF A NEW PHENOTYPE $$$

Isn't it amazing that in the above report from Ryan A. Maddox, MPH Epidemiologist CJD 2011 and the 9th Annual CJD Foundation Family Conference July 10, 2011, none of the information about what the rest of the world is worried about, this new threat from the atypical TSE's. USA Typical and Atypical BSE, USA Typical and Atypical Scrapie, and USA Typical and Atypical Chronic Wasting Disease as having zoonotic potential. The USDA, FDA, CDC et al are still relying on science that is almost 30 years old i.e. the UKBSEnvCJD only theory.

stupid is, as stupid does, and some times you just can't fix stupid $$$

TSS

DEEP THROAT TO TSS 2000-2001 (take these old snips of emails with how ever many grains of salt you wish. ...tss)

The most frightening thing I have read all day is the report of Gambetti's finding of a new strain of sporadic cjd in young people...Dear God, what in the name of all that is holy is that!!! If the US has different strains of scrapie.....why????than the UK...then would the same mechanisms that make different strains of scrapie here make different strains of BSE...if the patterns are different in sheep and mice for scrapie.....could not the BSE be different in the cattle, in the mink, in the humans.......I really think the slides or tissues and everything from these young people with the new strain of sporadic cjd should be put up to be analyzed by many, many experts in cjd........bse.....scrapie Scrape the damn slide and put it into mice.....wait.....chop up the mouse brain and and spinal cord........put into some more mice.....dammit amplify the thing and start the damned research.....This is NOT rocket science...we need to use what we know and get off our butts and move....the whining about how long everything takes.....well it takes a whole lot longer if you whine for a year and then start the research!!! Not sure where I read this but it was a recent press release or something like that: I thought I would fall out of my chair when I read about how there was no worry about infectivity from a histopath slide or tissues because they are preserved in formic acid, or formalin or formaldehyde.....for God's sake........ Ask any pathologist in the UK what the brain tissues in the formalin looks like after a year.......it is a big fat sponge...the agent continues to eat the brain ......you can't make slides anymore because the agent has never stopped........and the old slides that are stained with Hemolysin and Eosin......they get holier and holier and degenerate and continue...what you looked at 6 months ago is not there........Gambetti better be photographing every damned thing he is looking at.....

Okay, you need to know. You don't need to pass it on as nothing will come of it and there is not a damned thing anyone can do about it. Don't even hint at it as it will be denied and laughed at.......... USDA is gonna do as little as possible until there is actually a human case in the USA of the nvcjd........if you want to move this thing along and shake the earth....then we gotta get the victims families to make sure whoever is doing the autopsy is credible, trustworthy, and a saint with the courage of Joan of Arc........I am not kidding!!!! so, unless we get a human death from EXACTLY the same form with EXACTLY the same histopath lesions as seen in the UK nvcjd........forget any action........it is ALL gonna be sporadic!!!

And, if there is a case.......there is gonna be every effort to link it to international travel, international food, etc. etc. etc. etc. etc. They will go so far as to find out if a sex partner had ever traveled to the UK/europe, etc. etc. .... It is gonna be a long, lonely, dangerous twisted journey to the truth. They have all the cards, all the money, and are willing to threaten and carry out those threats....and this may be their biggest downfall...

Thanks as always for your help. (Recently had a very startling revelation from a rather senior person in government here..........knocked me out of my chair........you must keep pushing. If I was a power person....I would be demanding that there be a least a million bovine tested as soon as possible and agressively seeking this disease. The big players are coming out of the woodwork as there is money to be made!!! In short: "FIRE AT WILL"!!! for the very dumb....who's "will"! "Will be the burden to bare if there is any coverup!"

again it was said years ago and it should be taken seriously....BSE will NEVER be found in the US! As for the BSE conference call...I think you did a great service to freedom of information and making some people feign integrity...I find it scary to see that most of the "experts" are employed by the federal government or are supported on the "teat" of federal funds. A scary picture! I hope there is a confidential panel organized by the new government to really investigate this thing.

You need to watch your back........but keep picking at them.......like a buzzard to the bone...you just may get to the truth!!! (You probably have more support than you know. Too many people are afraid to show you or let anyone else know. I have heard a few things myself... you ask the questions that everyone else is too afraid to ask.)

3. Prof. A Robertson gave a brief account of BSE. The US approach was to accord it a very low profile indeed. Dr. A Thiermann showed the picture in the ''Independent'' with cattle being incinerated and thought this was a fanatical incident to be avoided in the US at all costs. BSE was not reported in the USA.

One reason for this was the _inaccuracy_ in coding of cases correctly certified as CJD Coding is carried out by staff who are not medically qualified and it is not surprising that coding errors occur in the processing of large numbers of certificates. In 1982, 12,000 certificates per week were processed at the office of population censuses and surveys bu 15 coders and 6 checkers (Alderson et al., 1983). The occurrence of both inter- and intra-observer coding errors has been described (Curb et al., 1983) and the _inaccuracies_ of BOTH certification and coding discovered in this study _support_ the introduction of a more accurate system of death certificates and a more detailed and specific coding system...

Draft Proposal For The Monitoring of Creutzfeldt-Kakob Disease 1989 Dr. R. Will

snip...

IDENTIFICATION OF CASES

Cases of CJD may be identified from death certificates, but this alone is unlikely to provide adequate monitoring. ERRORS are made in certification and diagnosis; in the Oxford study death certificates were obtained on a series of known confirmed cases and CJD was mentioned in only 66% of certificates. In another series of 175 certified cases, 42 patients were judged not to have suffered from CJD after examination of case notes (7)...

In collaboration with appropriate local and state health departments and the National Prion Disease Pathology Surveillance Center, CDC is facilitating or conducting such surveillance and case- investigations, including related laboratory studies to characterize CJD and CWD prions.

In addition, the NPDPSC sees less than half of all the CJD cases each year, so the CDC's investigational system not only is missing many of the misdiagnosed CJD cases, it also is not conducting autopsies on most of the detected cases.

[ProMED-mail thanks Terry S Singeltary Sr for drawing attention to this comprehensive document which provides a current evaluation of experimental work designed to explore the zoonotic potential of the various recently recognised TSEs of domestic and other animals. It is concluded that at present the only TSE agent demonstrated to be zoonotic is the classical BSE agent. Nor can it be entirely excluded at the present time that a small proportion of cases of sporadic CJD may be environmentally acquired. - Mod.CP]

Please notice where sporadic CJD cases in 1996 went from 28 cases, to 215 cases in 2009, the highest recorded year to date. sporadic CJD is on a steady rise, and has been since 1996.

I also urge you to again notice these disturbing factors in lines 5 and 6 ;

5 Includes 18 cases in which the diagnosis is pending, and 18 inconclusive cases;

6 Includes 23 (22 from 2010) cases with type determination pending in which the diagnosis of vCJD has been excluded.

========end=====tss=====2011

UPDATE JULY 2011 MORE OF THE "PENDING CLASSIFICATION CREUTZFELDT JAKOB DISEASE'' STEADY INCREASING...TSS

case; 5 Includes 13 cases in which the diagnosis is pending, and 18 inconclusive cases; 6 Includes 18 (15 from 2011) cases with type determination pending in which the diagnosis of vCJD has been excluded.

Manuscript Draft Manuscript Number: Title: HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory Article Type: Personal View Corresponding Author: Mr. Terry S. Singeltary, Corresponding Author's Institution: na First Author: Terry S Singeltary, none Order of Authors: Terry S Singeltary, none; Terry S. Singeltary Abstract: TSEs have been rampant in the USA for decades in many species, and they all have been rendered and fed back to animals for human/animal consumption. I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2007.

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

CHAPTER 14

Laying Odds

Are prion diseases more prevalent than we thought?

Researchers and government officials badly underestimated the threat that mad cow disease posed when it first appeared in Britain. They didn't think bovine spongiform encephalopathy was a zoonosis-an animal disease that can sicken people. The 1996 news that BSE could infect humans with a new form of Creutzfeldt-Jakob disease stunned the world. It also got some biomedical researchers wondering whether sporadic CJD may really be a manifestation of a zoonotic sickness. Might it be caused by the ingestion of prions, as variant CJD is?

Revisiting Sporadic CJD

It's not hard to get Terry Singeltary going. "I have my conspiracy theories," admitted the 49-year-old Texan.1 Singeltary is probably the nation's most relentless consumer advocate when it comes to issues in prion diseases. He has helped families learn about the sickness and coordinated efforts with support groups such as CJD Voice and the CJD Foundation. He has also connected with others who are critical of the American way of handling the threat of prion diseases. Such critics include Consumers Union's Michael Hansen, journalist John Stauber, and Thomas Pringle, who used to run the voluminous www.madcow. org Web site. These three lend their expertise to newspaper and magazine stories about prion diseases, and they usually argue that prions represent more of a threat than people realize, and that the government has responded poorly to the dangers because it is more concerned about protecting the beef industry than people's health.

Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

"My name is Terry S Singeltary Sr, and I live in Bacliff, Texas. I lost my mom to hvCJD (Heidenhain variant CJD) and have been searching for answers ever since. What I have found is that we have not been told the truth. CWD in deer and elk is a small portion of a much bigger problem." 49-year-old Singeltary is one of a number of people who have remained largely unsatisfied after being told that a close relative died from a rapidly progressive dementia compatible with spontaneous Creutzfeldt-Jakob disease (CJD). So he decided to gather hundreds of documents on transmissible spongiform encephalopathies (TSE) and realised that if Britons could get variant CJD from bovine spongiform encephalopathy (BSE), Americans might get a similar disorder from chronic wasting disease (CWD)-the relative of mad cow disease seen among deer and elk in the USA. Although his feverish.

To the Editor: In their Research Letter, Dr Gibbons and colleagues1 reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD) has been stable since 1985. These estimates, however, are based only on reported cases, and do not include misdiagnosed or preclinical cases. It seems to me that misdiagnosis alone would drastically change these figures. An unknown number of persons with a diagnosis of Alzheimer disease in fact may have CJD, although only a small number of these patients receive the postmortem examination necessary to make this diagnosis. Furthermore, only a few states have made CJD reportable. Human and animal transmissible spongiform encephalopathies should be reportable nationwide and internationally.

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States

26 March 2003

Terry S. Singeltary, retired (medically) CJD WATCH

I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?

An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.

Methods:

12 years independent research of available data

Results:

I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.

Conclusion:

I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.

CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

"Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle."